Thiopurines (TPRs) are effective drugs in treating neuromyelitis optica spectrum disorders (NMOSD) and other diseases. TPRs' toxicity is mainly imputed to thiopurine S-methyltransferase (TPMT) activity. In Chinese population, the most common and important variation of TPMT is TPMT*3C (rs1142345). This study aims to reveal the association between TPMT activity and genetic polymorphisms of TPMT in patients with NMOSD in China. An LC-MS/MS method was used to evaluate the TPMT activity by using 6-mercapthioprine as substrate in human erythrocyte hemolysate via 1 h incubation at 37 °C to form its methylated product 6-methylmercaptopurine (6-MMP)...

Objective: To evaluate the clinical characteristics and the corresponding MRI and laboratory findings in patients with neuromyelitis optica spectrum disorder (NMOSD) associated with area postrema (AP). Methods: The study was a retrospective analysis of data from 120 NMOSD patients, and 18 cases were with AP out of these patients, The clinical presentation, MRI changes, serological markers and treatment outcome were reported. Results: AP occurred in 18 patients (15%, 18/120). AP was the onset symptom in 14 (14/18) patients and 3 days to 7 months (median 40 days) later, optic neuritis or myelitis was involved...

Neuromyelitis optica spectrum disorders (NMOSD) are mostly relapsing inflammatory disorders of the central nervous system (CNS). Optic neuritis (ON) is the first NMOSD-related clinical event in 55% of the patients, which causes damage to the optic nerve and leads to visual impairment. Retinal optical coherence tomography (OCT) has emerged as a promising method for diagnosis of NMOSD and potential individual monitoring of disease course and severity. OCT not only detects damage to the afferent visual system caused by ON but potentially also NMOSD-specific intraretinal pathology, i...

OBJECTIVE: To assess the value of the central vein sign (CVS) on a clinical 3T scanner to distinguish between multiple sclerosis (MS) and neuromyelitis optica spectrum disorder (NMOSD). METHODS: Eighteen aquaporin-4-antibody-positive patients with NMOSD, 18 patients with relapsing-remitting MS, and 25 healthy controls underwent 3T MRI. The presence of a central vein in white matter lesions on susceptibility-weighted imaging, defined as a thin hypointense line or a small dot, was recorded...

OBJECTIVES: We aimed to evaluate the utility of the recently described brain lesion distribution criteria to differentiate multiple sclerosis (MS) from aquaporin-4 immunoglobulin G-positive neuromyelitis optica spectrum disorder (NMOSD) and myelin oligodendrocyte glycoprotein immunoglobulin G-associated encephalomyelitis (MOG-EM) at disease onset in an Asian cohort. METHODS: A total of 214 patients who fulfilled the published criteria for MS, NMOSD, or MOG-EM and underwent brain magnetic resonance imaging (MRI) within 3 months of disease onset were enrolled...

RATIONALE: Longitudinally extensive transverse myelitis (LETM) is characterized by contiguous inflammatory lesions of spinal cord extending to ≥3 vertebral segments. The etiology of LETM is complicated, including various infection, autoimmune disease, and so on. Neuromyelitis optic spectrum disorder (NMOSD) is the most common cause of LETM. Several case reports have suggested the associations between NMOSD and pulmonary tuberculosis (PTB). PATIENT CONCERNS: Patient 1, a 20-year-old woman who had a past history of PTB, presented with weakness, numbness, and pain in the limbs...

Rituximab has been effectively used for treating neuromyelitis optica spectrum disorder (NMOSD) for several years. However those regimens exert a heavy burden on Chinese patients. The aim of our study was to investigate an effectiveness, economic alternatives of RTX. The enrolled patients received different immunosuppressant drugs. Annual relapse rate (ARR), neurological disability (Expanded Disability Status Scale, EDSS), time to the next relapse were evaluated after treatments. Fourteen patients treated with RTX and 37 relapse events from 23 patients treated with traditional immunosuppressant drugs (ISDs) were analyzed in our study...

Neuromyelitis optica spectrum disorder (NMOSD) has unknown risk factors. The aim of this study was to identify the environmental risk factors for NMOSD. A case-control study was conducted in Tehran from 2015 to 2016 among 100 patients with NMOSD. Sex-matched healthy controls (n = 400) were selected through random digit dialing (RDD). Logistic regression was used to estimate unadjusted and adjusted ORs (odds ratio) at 95% confidence intervals (CI) via SPSS. Compared with the control population, in NMOSD patients, the adjusted OR for low dairy consumption per week was (OR = 18...

BACKGROUND: Short transverse myelitis (STM) is considered uncommon in neuromyelitis optica spectrum disorders (NMOSD). Poor recognition of STM occurring in NMOSD may lead to increased delay in diagnosis and appropriate treatment. OBJECTIVES: The aim of this study was to assess the frequency and characteristics of STM in Chinese patients with NMOSD. METHODS: We enrolled 91 patients with NMOSD based on the 2015 International Consensus Diagnostic Criteria for NMOSD...

We present a case of a woman with systemic lupus erythematosus (SLE) who had refractory episodes of neuromyelitis optica spectrum disorder (NMOSD) and was successfully treated with rituximab. She was positive for anti-aquaporin-4 (AQP4) antibody and had typical cranial and longitudinally extended spinal lesions but no optic nerve involvement. There is no established treatment for NMOSD/SLE overlap cases. Our experience suggests that rituximab may be effective for patients with combined SLE and anti-AQP4 antibody-positive NMOSD...

Background: A prominent pathological feature of neuromyelitis optica spectrum disorders (NMOSD) is markedly greater eosinophilic infiltration than that seen in other demyelinating diseases, like multiple sclerosis (MS). Eosinophils express the chemokine receptor CCR3, which is activated by eotaxins (CCL11/eotaxin-1, CCL24/eotaxin-2, CCL26/eotaxin-3) and CCL13 [monocyte chemoattractant protein (MCP)-4]. Moreover, CCL13 is part of the chemokine set that activates CCR2. The present study aimed to evaluate plasma levels of eotaxins (CCL11, CCL24, and CCL26) and MCPs (CCL13, CCL2, CCL8, and CCL7) in patients with NMOSD during remission...

Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune inflammatory disorder of the central nervous system (CNS). Serum immunoglobulin G autoantibodies (NMO-IgG) are identified in the majority of NMOSD patients. The Pediatric form presents before 18 years. Based on the similarity of clinical, neuroimaging, and laboratory characteristics of pediatric NMOSD to those of the adult form, the international panel suggested that adult criteria of NMOSD also are appropriate in pediatric patients. However, the proposed criteria need validation in pediatric patients...

Dengue virus infection is a disease with high incidence in some tropical and subtropical countries. A variety of neurological complications of dengue fever (DF) has been described including two cases with the phenotype of neuromyelitis optica spectrum disorder (NMOSD). However, aquaporin-4 serostatus was unknown or negative in these patients. We report two patients with NMOSD occurring in association with DF. The first patient presented with brainstem symptoms and the second one with isolated unilateral optic neuritis...

PURPOSE OF REVIEW: Neuromyelitis optica spectrum disorders (NMOSD) are severe inflammatory diseases of the central nervous system (CNS), with the presence of aquaporin 4 (AQP4)-specific serum antibodies in the vast majority of patients, and with the presence of myelin oligodendrocyte glycoprotein (MOG)-specific antibodies in approximately 40% of all AQP4-antibody negative NMOSD patients. Despite differences in antigen recognition, the preferred sites of lesions are similar in both groups of patients: They localize to the spinal cord and to the anterior visual pathway including retina, optic nerves, chiasm, and optic tracts, and - to lesser extent - also to certain predilection sites in the brain...

In the short time since 2014, three pivotal, worldwide studies in neuromyelitis optica spectrum disorders have been launched: eculizumab, SA237 and inebelizumab, each based on a unique mechanism. Areas covered: In this review, we provide a discussion on the trial data available for each drug, a brief description of the trial design, and our expert opinion on the potential benefits and risks. Expert opinion: Eculizumab, a C5 complement inhibitor, may prove useful in the treatment of intractable cases of NMOSD, but physicians must be aware of the known risk of meningococcal infection...

Differential diagnosis for neuromyelitis optica spectrum disorder (NMOSD) and multiple sclerosis (MS) is always doubtful. To differentiate these diseases, we studied the immune status in the blood of patients with MS (n = 45) or NMOSD (n = 23) at remission phase. Remitting NMOSD patients had increased levels of CXCL13 and memory B cells, while remitting MS patients had elevated levels of galectin-9 and Th1 cells. A diagnostic model with these four variables is built to distinguish remitting NMOSD from MS with a sensitivity of 91...